For example a rapidly progressive vasculopathy is described in the caveolin-1 knockout mouse, which occurs in part because of uncontrolled endothelial proliferation, alterations in vasomotor tone, and a fibrotic phenotype associated with increased signaling through the TGF-β axis [27,28], and second, the Tβ RICA Cre-ER mouse strain in which constitutive activation of the Tβ RI in fibroblasts results in fibrotic thickening of small vessels in the lung and kidney but histologically normal large vessels and heart [29]. Here, CAV1 is linked to vascular disorder.